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. 2021 May 5;16:201. doi: 10.1186/s13023-021-01823-3

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© The Author(s) 2021

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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Fig. 4 — Expression levels of lipoyl proteins, Fe-S cluster complex proteins and mitochondrial complex I subunits in mutant PANK2 fibroblasts. Control (C) and PKAN fibroblasts (P1 and P3) were treated with 500 μM pantothenate for 20 days. a Immunoblotting analysis of cellular extracts from Control (C) and patients P1, P2 and P3 fibroblasts. Protein extracts (50 μg) were separated on a SDS polyacrylamide gel and immunostained with antibodies against lipoic acid, Fe-S cluster complex proteins NFS1, ISD11 and LYRM4 and mitochondrial complex I subunits MT-ND1 and NDUFA9. b Densitometry of the Western blotting. Data represent the mean ± SD of three separate experiments. *p < 0.01 between PKAN patients and controls; ^ap < 0.01 between untreated and treated cells. A.U., arbitrary units. R = responder fibroblasts